Zamboni G, Antoniazzi F, Tatò L
Clinica Pediatrica, Università di Verona, Italia.
Pediatr Med Chir. 1996 Jan-Feb;18(1):3-12.
Sex determination and differentiation is a sequential process that involves genetic, gonadal, phenotypic and psychological sex. Sex determination is primarily testis determination. The primary event is differentiation of the gonad; all subsequent sexual differentiation is hormonally controlled. The Authors present a classification of abnormal sexual development and consider the errors of primary sex differentiation (anomalies of sex chromosomes and of gonadogenesis) and the errors of sexual differentiation (inadequate masculinization of genetic male and virilization of genetic female). At the end the clinical approach to disorders of sexual differentiation is briefly considered.
性别决定与分化是一个连续的过程,涉及遗传、性腺、表型和心理性别。性别决定主要是睾丸决定。首要事件是性腺的分化;所有后续的性分化均受激素控制。作者提出了异常性发育的分类,并考虑了原发性性别分化的错误(性染色体和性腺发生异常)以及性分化的错误(遗传男性的男性化不足和遗传女性的男性化)。最后简要讨论了性分化障碍的临床处理方法。